The term congenital nephrotic syndrome (CNS) refers to disease that is present at birth or within the first three months of life. Later onset, between three months and one year of age, is called infantile nephrotic syndrome. Most of these children have a genetic basis for the renal disease and a poor outcome. The precise diagnosis of the glomerular lesion is based on clinical, laboratory, and histological criteria.

The causes of congenital and infantile nephrotic syndrome will be discussed here.

ETIOLOGY

The results of four reports indicate that mutations in five different genes are responsible for more than 80 percent of congenital nephrotic syndrome (CNS) cases (table 1).

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